Lymphomatoid papulosis treated with extracorporeal photochemotherapy

Lymphomatoid papulosis (LP) is a rare low-grade T-cell lymphoma which may respond to cytotoxic drugs and PUVA irradiation but long-term remission has not been achieved. Extracorporeal photochemotherapy (ECP) is an immunomodulating therapy used successfully for several types of CTCL, no experience with LP has been reported yet. ECP therapy with 8-methoxypsoralen was introduced on two subsequent days once per month for half a year in a 42-year old women with a 20-year history of LP. Disseminated papules disappeared rapidly after 3 cycles of ECP treatment but metastatic spread continued, which made necessary a subsequent polychemotherapy. One year later, the patient died of central nervous system metastasis. ECP monotherapy seems unable to control disease progression in LP despite beneficial effects on skin lesions.